Abnormal head shape

What is meant by abnormal head shape?

An abnormally shaped head is usually recognized at birth. There are three causes of abnormal head shape in infants.

The largest group of infants with an abnormal head shape is those who have positional deformities which develop during pregnancy or while sleeping.

The next most common group is those infants who present with early closure of the cranial sutures (craniosynostosis).

The smallest group are infants with craniofacial syndromes, such as Apert's, Crouzon's, and Pfeiffer's.

What congenital syndromes cause abnormal head shape?

A small number of children who present with abnormal head shape are born with identifiable syndromes, such as Crouzon's or Apert's. These children have craniosynostosis and facial bone abnormalities as well as deformities of the hands and feet. They usually require surgery to correct the skull prior to one year of age, and at age six to eight years of age to have the facial bones moved. Finally, in adolescence, they will again require surgery on the face and jaw.

What is craniosynostosis and how does it cause abnormal head shape?

Diagram of the fontanelles and sutures in the skull. The sagittal suture runs from front to back, the two coronal sutures run down the sides, and the metopic suture runs down the front of the forhead. Early closure of any one or more of these sutures will result in an abnormal head shape, as shown below.

Craniosynostois is the premature (early) closure of one or more cranial sutures. These children present with an abnormal head shape that varies according to the suture involved. Those with sagittal suture involvement have long, thin skulls, while those with coronal suture involvement have a flat, short head on one or both sides, depending on whether one or both coronal sutures are involved. Children born with metopic craniosynostosis have narrow, pointed foreheads.

scaphocephaly
Diagram showing scaphocephaly, a long, narrow head shape, often due to premature closure of the sagittal suture. Surgical treatment involves removing bone from the midline sagittal suture.

plagiocephaly
Diagram showing plagiocephaly, an asymmetric head shape often resulting from premature closure of one of the coronal sutures (note in plagiocephaly that the suture on the opposite side is normal). Surgical treatmant involves removing bone from the affected coronal suture.

Surgery is ideally performed before nine months of age. In children with multiple affected sutures or with sagittal craniosynostosis, surgery is performed prior to three months of age. Surgery involves removing the fused suture and repositioning the skull and/or face.

How do positional deformities cause abnormal head shape?

Infants with positional deformities may present with a number of different head shapes. They may have unilateral flattening of their posterior skull, which is plagiocephaly, and/or forehead bulging along with bilateral flattening, which is brachycephaly. When they have increased skull height and/or a long, narrow skull it is scaphocephaly. Deformities result from positioning during pregnancy, sleeping position, or from neck tightness. An asymmetrical skull can result in asymmetries of the face causing various functional problems that affect chewing, speech, breathing, and vision. Some of these infants can be managed with just positioning, such as changing their sleeping position. If the deformity persists, however, treatment may be necessary.

Infant treatment of positional deformities is with a molding cap, a dynamic orthotic cranioplasty (sm) (DOC) BandŽ. Ideally, the treatment is begun during the first six months of life. The band is worn 23 hours a day and requires 3-4 months of wear. Severe cases in older children require longer periods of correction. The result of wearing the DOC BandŽ is typically a 70 percent rate of improvement, but the correction rate correlates with the severity of the deformity and the age that treatment was commenced. Long-term follow up in these children reveals that the correction does not relapse with further growth. (sm: Cranial Technologies, Inc. 1-800-689-3402).

This information is provided to help you better understand craniofacial deformities. If you have further questions, please don't hesitate to ask your child's physician. Please be assured that physicians at Hermann Children's Hospital and the University of Texas Medical School at Houston provide your child the latest, most compassionate medical care.